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A newly recognized feature of ectrodactyly, ectodermal dysplasia, clefting (EEC) syndrome: comedone naevus.
Dermatologica. 1984; 169(2):80-5.D

Abstract

A 21-year-old Black South African man with clinical features of ectrodactyly, ectodermal dysplasia, cleft lip/palate (EEC) syndrome is described. The patient had the following anomalies: hypoplastic, peg-shaped teeth; fine, silky hair; absent lacrimal puncta resulting in secondary photophobia, blepharitis and corneal opacities. The hands and feet showed absent digits, symphalangism and displacement of bone consistent with ectrodactyly. In addition, the patient had extensive comedone naevus, a feature not previously reported in EEC syndrome. We stress that the dysplastic ectodermal features of EEC syndrome are not typical of either hidrotic or anhidrotic ectodermal dysplasia but fall into a class of their own. Clefting of the lip and palate were absent due, it is postulated, to the variable expressivity of EEC syndrome.

Authors

No affiliation info availableNo affiliation info available

Pub Type(s)

Case Reports
Journal Article

Language

eng

PubMed ID

6479416

Citation

Leibowitz, M R., and T Jenkins. "A Newly Recognized Feature of Ectrodactyly, Ectodermal Dysplasia, Clefting (EEC) Syndrome: Comedone Naevus." Dermatologica, vol. 169, no. 2, 1984, pp. 80-5.
Leibowitz MR, Jenkins T. A newly recognized feature of ectrodactyly, ectodermal dysplasia, clefting (EEC) syndrome: comedone naevus. Dermatologica. 1984;169(2):80-5.
Leibowitz, M. R., & Jenkins, T. (1984). A newly recognized feature of ectrodactyly, ectodermal dysplasia, clefting (EEC) syndrome: comedone naevus. Dermatologica, 169(2), 80-5.
Leibowitz MR, Jenkins T. A Newly Recognized Feature of Ectrodactyly, Ectodermal Dysplasia, Clefting (EEC) Syndrome: Comedone Naevus. Dermatologica. 1984;169(2):80-5. PubMed PMID: 6479416.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - A newly recognized feature of ectrodactyly, ectodermal dysplasia, clefting (EEC) syndrome: comedone naevus. AU - Leibowitz,M R, AU - Jenkins,T, PY - 1984/1/1/pubmed PY - 1984/1/1/medline PY - 1984/1/1/entrez SP - 80 EP - 5 JF - Dermatologica JO - Dermatologica VL - 169 IS - 2 N2 - A 21-year-old Black South African man with clinical features of ectrodactyly, ectodermal dysplasia, cleft lip/palate (EEC) syndrome is described. The patient had the following anomalies: hypoplastic, peg-shaped teeth; fine, silky hair; absent lacrimal puncta resulting in secondary photophobia, blepharitis and corneal opacities. The hands and feet showed absent digits, symphalangism and displacement of bone consistent with ectrodactyly. In addition, the patient had extensive comedone naevus, a feature not previously reported in EEC syndrome. We stress that the dysplastic ectodermal features of EEC syndrome are not typical of either hidrotic or anhidrotic ectodermal dysplasia but fall into a class of their own. Clefting of the lip and palate were absent due, it is postulated, to the variable expressivity of EEC syndrome. SN - 0011-9075 UR - https://wwww.unboundmedicine.com/medline/citation/6479416/A_newly_recognized_feature_of_ectrodactyly_ectodermal_dysplasia_clefting__EEC__syndrome:_comedone_naevus_ L2 - http://www.diseaseinfosearch.org/result/2437 DB - PRIME DP - Unbound Medicine ER -